Use of iris recognition camera technology for the quantification of corneal opacification in mucopolysaccharidoses

Aslam, Tariq; Shakir, Savana; Wong, James; Au, Leon; Ashworth, Jane

doi:10.1136/bjophthalmol-2011-300996

Cited by 15 publications

(25 citation statements)

References 5 publications

(4 reference statements)

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“…The COM scores, however, also may be influenced by other corneal disease states. 8 This can be seen in patient 6 whose left eye had an increase in COM score due to worsening of corneal clouding due to secondary glaucoma requiring subsequent surgery. The left eye was excluded from COM score analyses as her large peripheral iridotomy meant the image was no longer standardized.…”

Section: Discussionmentioning

confidence: 99%

“…This study demonstrated that new methods to quantify corneal clouding 8,13 are of use in monitoring changes over time and effect of treatment. The technique of iris camera assessment of corneal clouding also may be applicable to other corneal pathologies resulting in opacification, such as corneal dystrophy, bullous keratopathy, and infective keratitis.…”

Section: Discussionmentioning

confidence: 99%

“…The COM score method has been validated and shown to have good repeatability in our earlier study. 8 The COM score is a semiautomated score that calculates the corneal opacity at each individual pixel and then provides a mean. The standard deviation demonstrates the spread of the corneal opacity pixel measurements throughout the image analyzed.…”

Section: Methodsmentioning

confidence: 99%

“…Retrospective case series of small numbers of patients in the literature, followed for short periods of time, have used subjective grading scales of corneal opacification resulting in difficulties in interpreting the results. 2,5 In vitro studies have demonstrated safe and efficient gene replacement in human corneal explants from MPSI patients, 6 and human umbilical mesenchymal stem cells have been shown to reduce GAG deposition and corneal haze when transplanted into the corneal stroma of MPSVII mice, 7 suggesting that these techniques may have future potential for treating the corneal clouding associated with MPS.In this study, we used a previously validated system of assessment using an Iris recognition camera, 8,9 to prospectively measure changes in corneal clouding in patients with MPS types I (Hurler) and VI (Maroteaux-Lamy) over time and to assess the effects of treatment with ERT and HSCT on corneal clouding. …”

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confidence: 99%

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Objective Quantification of Changes in Corneal Clouding Over Time in Patients With Mucopolysaccharidosis

Javed

Aslam

Jones

et al. 2017

Invest. Ophthalmol. Vis. Sci.

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Citation: Javed A, Aslam T, Jones SA, Ashworth J. Objective quantification of changes in corneal clouding over time in patients with mucopolysaccharidosis. Invest Ophthalmol Vis Sci. 2017;58:954-958. DOI:10.1167/ iovs.16-20647 PURPOSE. We determine objective changes in corneal opacification levels over time in patients with mucopolysaccharidoses (MPS) treated with enzyme replacement therapy or hematopoietic stem cell transplant. A prospective cohort study was done of 9 patients with MPS I (Hurler) or VI (Maroteaux-Lamy).METHODS. Quantification of corneal clouding using the Iris camera and full ophthalmic examination, including subjective assessment of corneal clouding, was done in 2011 and repeated in 2015/2016. Patients also had assessment of biomarkers, including dermatan sulfate/chondroitin sulfate (DS/CS) ratio. Change in corneal opacification were measured by Iris camera corneal opacification measure (COM) score during a mean of 60 months followup. RESULTS.A total of 5/17 (29%) eyes had a deterioration in COM score, indicating increased corneal clouding. There was no significant change in COM score in 10/17 (59%) patient eyes. One patient (2/17 eyes) demonstrated significant improvement in corneal clarity and this was associated with improved biomarker levels.CONCLUSIONS. Assessment of COM scores using the Iris camera are an objective means of monitoring corneal opacification over time in patients with MPS. Corneal opacification may potentially be reversed with intensive treatment demonstrated by impact on biomarkers.Keywords: mucopolysaccharidoses, paediatric ophthalmology, metabolic medicine T he mucopolysaccharidoses (MPS) are a group of rare metabolic diseases characterized by defects of specific lysosomal enzymes involved in the degradation of glycosaminoglycans (GAGs). Glycosaminoglycan deposition in multiple tissues and organs results in a wide range of systemic manifestations, including dysmorphic facial features, vision and hearing impairment, cardiorespiratory problems, joint and bone diseases, neurologic problems, and intellectual impairment. Corneal opacification is an early clinical feature in several of the MPS subtypes (MPSI Hurler and Hurler-Scheie, MPSIVA Morquio, MPSVI Maroteaux-Lamy, MPSVII Sly), and can result in significant visual impairment.1 In addition, complications, such as retinopathy, glaucoma, and optic neuropathy, may contribute to visual loss in patients with MPS. Current treatment options for MPS include enzyme replacement therapy (ERT), which is available for MPS I, II, IVA, and VI; and hematopoietic stem cell transplantation (HSCT), which is useful for selected patients with MPS types I and VI.1,2 The untreated clinical course of corneal clouding in MPS is thought to be one of gradual deterioration, but the extent and speed of deterioration have not been documented. Enzyme replacement therapy is known to be effective in improving the systemic manifestations of MPS in types I, II, IVA, and VI by improving respiratory function and stamina, and improving quality of life.2...

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

mentioning

confidence: 99%

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Objective Quantification of Changes in Corneal Clouding Over Time in Patients With Mucopolysaccharidosis

Javed

Aslam

Jones

et al. 2017

Invest. Ophthalmol. Vis. Sci.

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“…Communicating hydrocephalus. Hearing loss [2], corneal opacities [54,55]. Hepatosplenomegaly, umbilical and inguinal hernias [39,40].…”

Section: Othersmentioning

confidence: 99%

Anesthetic Management in Mucopolysaccharidoses

Spinello

Novello

Pitino

et al. 2013

ISRN Anesthesiology

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Mucopolysaccharidoses (MPSs) are a group of uncommon genetic diseases of connective tissue metabolism. It is well established that the elective treatment of subjects affected by MPS is multidisciplinary and must be carried out by experienced personnel in highly specialist centers. However, there is the possibility to perform an anesthesia in a peripheral center, where anesthesiologists might not have a large experience of MPS. Various attempts to produce guidelines have been made for MPS. There has been an increasing interest in the topic as it is demonstrated by the number of scientific studies published in the last few years (peak in 2011). We want to provide a summary of anesthetic management, reviewing the current literature on the topic in a procedural algorithm for these high-risk patients, who require surgical procedures and diagnostic examinations under sedation with a higher frequency than the general population.

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Biomarkers of Glycosaminoglycans (GAG) accumulation in patients with mucopolysaccharidosis type VI—LeukoGAG, Corneal Opacification (COM) and Carotid Intima Media Thickening (CIMT)

Use of iris recognition camera technology for the quantification of corneal opacification in mucopolysaccharidoses

Cited by 15 publications

References 5 publications

Objective Quantification of Changes in Corneal Clouding Over Time in Patients With Mucopolysaccharidosis

Objective Quantification of Changes in Corneal Clouding Over Time in Patients With Mucopolysaccharidosis

Anesthetic Management in Mucopolysaccharidoses

Biomarkers of Glycosaminoglycans (GAG) accumulation in patients with mucopolysaccharidosis type VI—LeukoGAG, Corneal Opacification (COM) and Carotid Intima Media Thickening (CIMT)

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